Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: A population-based study.

OBJECTIVE: Evaluate the neonatal management and outcomes of neonates with prenatal diagnosis of esophageal atresia (EA) type A. METHODS: This population-based study was conducted using data from the French National Register for infants with EA born from 2008 to 2014, including all cases of EA type A. We compared prenatal and neonatal characteristics and outcomes in children with prenatal diagnosis of EA type A with those with a postnatal diagnosis until the age of 1. RESULTS: A total of 1118 live births with EA were recorded among which 88 (7.9%) were EA type A. Prenatal diagnoses were performed in 75 cases (85.2%), and counselling with a prenatal specialist was conducted in 84.8% of the prenatal group. Still within that group, the gestational age at delivery was significantly higher than in the postnatal group (36 [35-38] versus 34 [32-36] weeks; P = .048). Inborn births were more frequent in the prenatal group (86.1% vs 7.7%, P < .0001), and mortality and outcome were similar in both groups. CONCLUSION: Prenatal diagnosis is high in EA type A, which enables to offer an antenatal parental counseling and which avoids postnatal transfers. Prognosis of EA types A does not appear to be influenced by the prenatal diagnosis.

Safety and efficacy of one-stage total laparoscopic treatment of common bile duct stones in children.

BACKGROUND: The purpose of this study is to confirm the effectiveness of total laparoscopic treatment of common bile duct (CBD) stones in children. METHODS: All children who were treated in our department for cholelithiasis were reviewed from 1996 to 2013. Data collection focused on children with CBD stones, including age, sex, symptoms at diagnosis, hepatic and pancreatic blood tests results, US scan results, etiology, detailed surgical technique, operative time, length of hospital stay, complications, and stone-free status or not, at last follow-up. RESULTS: 551 children were treated for cholelithiasis and had undergone laparoscopic cholecystectomy. Among those, 36 children (6.5%) presented with CBD stones with a mean age at symptom onset of 10.4 years (min-max: 4 months-18 years). A majority of the patients presented with hemolytic disease (61%). In 55% of the cases, cholangiography alone or simple serum saline flush of the biliary tree was sufficient to obtain a stone-free CBD. Additional maneuvers with Dormia basket or Fogarty catheter led to 72% of success rate. In 9 cases (25%) of failure of the procedure, 6 patients underwent endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy (ES), 1 patient was re-operated at day1 for hemorrhage, and 2 patients were followed by US scan with spontaneous evacuation of CBD stones. Mean follow-up was of 2 years (min-max: 1 month-5 years). All patients were stone free at last clinical and radiological evaluation. CONCLUSION: A one-stage total laparoscopic treatment of common bile duct stones in children is a safe, feasible, reproducible, and efficient procedure in 72% of the cases. This rate could be upgraded by a combination of laparoscopic and endoscopic technique during the same anesthesia and preserving Oddi sphincter function. These minimal invasive techniques still need to be developed in children.

Postoperative decrease in plasma sodium concentration after infusion of hypotonic intravenous solutions in neonatal surgery.

BACKGROUND: Hypotonic i.v. solutions can cause hyponatraemia in the context of paediatric surgery. However, this has not been demonstrated in neonatal surgery. The goal of this study was to define the relationship between infused perioperative free water and plasma sodium in neonates. METHODS: Newborns up to 7 days old undergoing abdominal or thoracic surgery were included in this prospective, observational study. Collected data included type and duration of surgery, calculated i.v. free water intake, and pre- and postoperative plasma sodium. Statistical analyses were performed using the Pearson correlation, Mann-Whitney test, and receiver operating characteristic analysis with a 1000 time bootstrap procedure. RESULTS: Thirty-four subjects were included. Postoperative hyponatraemia occurred in four subjects (11.9%). The difference between preoperative and postoperative plasma sodium measurements (ΔNaP) correlated with calculated free water intake during surgery (r=0.37, P=0.03), but not with preoperative free water intake. Calculated operative free water intake exceeding 6.5 ml kg(-1) h(-1) was associated with ΔNaP≥4 mM with a sensitivity and specificity [median (95% confidence interval)] of 0.7 (0.9-1) and 0.5 (0.3-0.7), respectively. CONCLUSIONS: Hypotonic solutions and i.v. free water intake of more than 6.5 ml kg(-1) h(-1) are associated with reductions in postoperative plasma sodium measurements ≥4 mM. In the context of neonatal surgery, close monitoring of plasma sodium is mandatory. Routine use of hypotonic i.v. solutions during neonatal surgery should be questioned as they are likely to reduce plasma sodium.

Oesophagogastric reconnection is possible.

INTRODUCTION: The first oesophagogastric dissociation was described by Bianchi in 1997 for the treatment of severe gastro-oesophageal reflux (GOR) in neurological patients to avoid serious respiratory complications. The dissociation leads to malabsorption and growth problems. We describe the first two cases of oesophagogastric reconnection long after lung growth. PATIENTS: Case no. 1 was a 12.7-year-old child with a history of type I oesophageal atresia. He required oesophagocoloplasty and gastric dissociation by the Bianchi procedure at the age of 3 months. He has oesophagogastric reconnection at 11 years old because he had a several macrocytic anaemia. Case no. 2 was also a 12-year-old boy, with an unlabelled multiple malformation syndrome with type I oesophageal atresia. He had an oesophagocoloplasty with gastric dissociation at the age of 3 months to protect his lungs. The reconnection was proposed at 10 years old because he developed multiple nutritional deficiencies and growth retardation. RESULTS: The two cases have no clinical symptom of reflux and their post-operative digestive contrasts are normal. Reconnection appears possible with or without preserving the Roux-en-Y loop. It improves absorption and corrects any vitamin deficiencies, allows the reconnected stomach to be monitored in terms of reflux and malignancy and enables enteral nutrition supplementation to be stopped. The possibility of reconnection after the Bianchi procedure therefore extends its indications, mostly in the case of oesophageal atresia complicated by severe GOR with pulmonary repercussions or tracheoesophageal cleft.

Acute chest syndrome after laparoscopic splenectomy in children with sickle cell disease: operative time dependent?

BACKGROUND: Laparoscopic splenectomy remains a technically demanding procedure. On patients with sickle cell disease (SCD), a post operative acute chest syndrome (ACS) can occur. The aim of the study was to look for predictive factors of post operative ACS. PATIENTS AND METHOD: It's a retrospective study on patients with SCD, who underwent a laparoscopic splenectomy in Robert Debré hospital, Paris, France, between March 2008 and December 2013. Diagnosis of ACS was done if the patient developed hypoxemia associated with fever above 38.5 °C and an infiltrate on chest x ray during the post operative course. Pre-, post- and operative factors were studied. Descriptive statistics were compared using the Mann-Whitney test or the exact Fisher test. A p inferior to 0.05 was considered as significant. RESULTS: 52 patients with SCD underwent a laparoscopic splenectomy. Twelve patients presented a post operative ACS (23%) (mean age at surgery 4 years old) while forty did not (mean age 5.25 years old). Neither previous episode of ACS nor any factors reflecting SCD severity were significant. The shorter the operative time was, the greater the risk of developing an ACS (p < 0.05). CONCLUSION: ACS is an important complication following laparoscopic splenectomy in patients with SCD. The immediate post operative management, in the absence of predictive factors for ACS, should be carefully followed in a high dependency unit at least for 48 h for all patients.

Paediatric and adolescent traumatic gastrointestinal injuries: results of a European multicentre analysis.

AIM: Paediatric gastrointestinal injuries (GIIs) are rare, and the aim of this multicentre study was to evaluate their outcomes in a large cohort. METHODS: Hospital databases of 10 European paediatric surgical centres were reviewed for paediatric traumatic GIIs managed between 2000-2010. RESULTS: Ninety-seven patients with a median age of 9 years (0-17 years) were identified, with 72 blunt and 25 penetrating GIIs. Initial diagnostics in 90 patients led to correct diagnosis in 71%. Diagnostics were delayed in 26 patients (median 24 h). Eighty-two patients required surgery (67 laparotomy, 12 laparoscopy and three other approaches). There was a 50% conversion in the laparoscopic group. Median hospital stay was 10 days (range 1-137 days), with longer duration influenced by associated injuries (n = 41). Diagnosis <24 h was associated with significantly shorter hospital stay compared to more than 24 h (p = 0.011). In one-third of patients, morbidities were not related to a diagnostic delay or type of injury. There were five lethal outcomes, four due to associated injuries. CONCLUSION: Initial diagnostics in traumatic paediatric GIIs provide false negatives in one-third of patients. Diagnostic delay <24 h is associated with a significantly shorter hospital stay. Although laparoscopy is associated with a conversion rate of 50%, it can be used for diagnosis in suspected cases to avoid nontherapeutic laparotomy.

Complications and Disease Recurrence After Ileocecal Resection in Pediatric Crohn's Disease: A Retrospective Study.

OBJECTIVE: The aim of this retrospective study was to describe the risk of postoperative recurrence (POR) after ileocecal resection, the occurrence of surgical complications, and identify predictors of these adverse postoperative outcomes in pediatric Crohn's disease (CD). PATIENTS AND METHODS: All the children less than 18 years of age with a diagnosis of CD, who underwent primary ileocecal resection for CD between January 2006 and December 2016 in our tertiary center, were considered for inclusion. Factors related to POR were investigated. RESULTS: A total of 377 children were followed for CD between 2006 and 2016. During this period, 45 (12%) children needed an ileocecal resection. POR was diagnosed in 16% (n = 7) at 1 year and 35% (n = 15) at the end of the follow-up, with a median follow-up of 2.3 years (Q1-Q3 1.8-3.3). Median duration of the postoperative clinical remission was 1.5 years (range 0.5-2). Multivariate Cox regression analysis identified only young age at diagnosis as a risk factor for POR.In total, 7 of the 43 patients (16%) developed severe postoperative complications, defined as requiring surgical, endoscopic, or radiological intervention. The only risk factor was intraoperative abscess. CONCLUSION: Only young age at diagnosis was associated with POR. This information could be useful to develop targeted therapeutic strategies for young CD children. At the end of follow-up with a median follow-up of 2.3 years (Q1-Q3 1.8-3.3), there was no surgical POR: endoscopic dilatation for POR should be considered in order to delay or prevent surgery.

Gastrografin for uncomplicated adhesive small bowel obstruction in children.

PURPOSE: The risk of bowel injury during surgery for small bowel obstruction (SBO) has generated interest in conservative treatment modalities. Few data are available on conservative Gastrografin treatment for SBO in children. METHODS: We prospectively included patients with uncomplicated adhesive SBO managed at a pediatric center between March 2009 and September 2010. Patients who were unimproved after 48 h of conservative treatment received 50-100 ml of Gastrografin. If Gastrografin was seen in the cecum on the abdominal radiograph 4-6 h later, feeding was initiated and the patient was discharged on the same day. Each patient was matched to 2 controls on the number of previous SBO episodes. The primary outcome was length of hospital stay (>3 days), and the secondary outcome was time from admission to first feed (>2 days). Both were compared in the two groups using conditional logistic regression. RESULTS: The 8 patients admitted for SBO were matched to 16 controls. Gastrografin administration was associated with significantly lower risks of staying in the hospital longer than 3 days (P < 0.10) and waiting more than 2 days before the first feed. CONCLUSION: This preliminary study suggests that Gastrografin may be useful for managing adhesive SBO in children.

Lessons learnt from two pediatric motor vehicle accidents resulting in anal canal, rectal and gluteal muscle wrenching.

Ano-rectal trauma is common in motor vehicle accidents involving children. Inadequate initial assessment of the extent of lesions may be life threatening. We describe two cases where children were struck by buses that subsequently rolled over them in the prone position, resulting in ano-rectal and gluteal muscle wrenching. The first patient was inadequately assessed. Initial management did not include a diverting stoma, leading to life-threatening necrosis and septic shock. The second benefitted from our previous experience and recovery was uneventful. The distinctive mechanism of trauma in true gluteal muscle and anal canal wrenching is discussed. Gluteal muscle, anal canal and rectal wrenching as a result of rolling force from a motor vehicle is a very serious condition requiring immediate intestinal diversion with a stoma. Immediate repair may be attempted at the same time as stoma creation if the patient is stable. Broad-spectrum antibiotics and close wound monitoring are necessary to avoid muscle necrosis and serious complications.

Congenital central hypoventilation syndrome and Hirschsprung disease: A retrospective review of the French National Registry Center on 33 cases.

AIM OF THE STUDY: Congenital Central Hypoventilation Syndrome (CCHS) is a rare affection associated to Hirschsprung disease (HD) in 20% of the cases. Using the French CCHS registry, we described the population of patients suffering from both CCHS and HD reporting the outcome on these patients. METHODS: Medical records were reviewed. Epidemiological, clinical, histological and genetic data were analyzed and extracted from the national French registry data. RESULTS: 33 patients had CCHS and HD. Thirty percent had a severe form of CCHS (Death owing to CCHS or 24/24 ventilation beyond 1 year old). Fifty four percent required tracheotomy. HD's pathologic segment was classic (Rectosigmoid and left colic form) in 20% and long (Above the splenic flexure) in 80%. Twenty four percent were treated with daily irrigation, 21% had colostomy without undergoing pullthrough, and 55% underwent optimal treatment (pull through). We failed to demonstrate a correlation between severity of CCHS and HD's length. The rate of mortality was 57% and was higher in the long HD group (p = 0.0005). Fourteen patients were still alive, aged 1 to 31 years old. Ninety two percent were weaned off the 24/24 ventilation. Regarding the intestinal function, 38% presented with soiling and 30% with chronic diarrhea. Hundred percent had CCHS follow-up while only 35% had no surgical follow-up in regard to the HD. CONCLUSIONS: This is the largest study regarding the CCHS / HD association and its long-term followup. Mortality is high demonstrating that a multidisciplinary follow-up on respiratory and intestinal function is necessary to improve outcome. Level III study.

Abdominal lymphangiomas in children: interest of the laparoscopic approach.

BACKGROUND: Lymphangiomas are rare benign lesions of the lymphatic system. The most common symptoms are abdominal tumor or "acute abdomen" in children. The treatment of choice is complete surgical resection, but the recurrence rate with incomplete resection is high, and laparotomy exposes the patient to adhesions. The authors report their experience with the lymphangioma laparoscopic approach. METHODS: This retrospective study examined 15 consecutive operations for lymphangiomas in children, ages 5 months to 14 years, treated during the 5-year period from 1999 to 2004. RESULTS: Six patients were treated using the primary laparotomy approach, and nine patients underwent the laparoscopic procedure, six successfully. Three conversions were necessary (1 case requiring partial colectomy, 1 retroperitoneal case with adherence on the aorta and vena cava, 1 case with partial volvulus). Morbidity included two cases of acute occlusion caused by adhesions after laparotomy. There was no recurrence of lymphangioma during a mean follow-up period of 35 months. CONCLUSION: The laparoscopy procedure could be used successfully for abdominal lymphangioma, even in an emergency. When the laparoscopic resection is impossible, laparotomy or sclerotherapy can be discussed.

Crohn's disease in children. Preliminary experience with a laparoscopic approach.

BACKGROUND: Laparoscopic surgery in patients with Crohn's disease (CD) has been demonstrated to have advantages over a conventional approach in children. The aim of this study was to review the children treated for CD with a laparoscopic approach, to report our indications, the surgical procedure, the complications, and to compare the children with pancolitis or ileocaecal (segmental) Crohn's disease. PATIENTS AND METHOD: We reviewed the files of 11 children treated for CD in a single institution between 1999 and 2004 for a retrospective study of clinical and surgical data. Mann-Whitney U-test was used for statistical analysis of nonparametric data. RESULTS: Eleven children were operated. The average age when initial clinical symptoms became apparent was 12.1 years (range 6.6 - 15), and surgery was performed after an average of 3.4 years of disease (range 1 - 7.6). The surgical indications were stenosis in 6 cases, failure to thrive in 1 case (segmental CD, SCD group) and pancolitis refractory to medical treatment in 4 cases (pancolitis group, PCD group). Mean operative time was 207 minutes (range 140 - 270) for the SCD group and 285 minutes (range 260 - 300) for the PCD group (p < 0.05). Three cases needed a conversion to open surgery (2 in PCD group, one in SCD group), mainly in relation to anastomosis performed with an EEA stapler. The average length of surgical unit stay was 6.5 days (range 4 - 8) for the PCD group and 6.4 days (range 4 - 8) for the SCD group; average follow-up was 16 months (range 3 - 38). Two patients had a relapse of CD (stenosis of the anastomosis in one, skin fistula in the other). CONCLUSION: A laparoscopic approach for ileocolic resection in Crohn's disease is a feasible procedure, even in cases of pancolitis. We recommend an extra-corporeal anastomosis because, in relation to the inflammatory bowel, the mechanical anastomosis is not a safe procedure in cases of pancolitis.

[Laparoscopic surgery of Meckel's diverticulum]. / Chirurgie coelioscopique du diverticule de Meckel.

Meckel's diverticulum is an evolution of omphalomesentric channel one of the most frequent embryologic abnormality of digestive tract. It is observed in two circumstances: during a routine exploration or after a complication: intestinal obstruction, peritonitis, recurrent abdominal pain. Only open or laparoscopic explorations allow a diagnosis. Resection of the diverticulum is recommended in children because the complications are frequent and serious. The preferred therapeutic method is the bowel resection with immediate anastomosis.

[Specificities of the treatment of abnormal pyelo-ureteral junction in children]. / Particularités du traitement de l'anomalie de la jonction pyélo-urétérale chez l'enfant.

Pyelo-ureteral junction abnormalities in children are most frequently discovered by prenatal ultrasound investigation. Most pyeloplasties by resection-anastomosis of the pyelo-ureteral junction are performed in young infants, usually with a posterior approach, patient prone. The indication and type of urinary pyelic drain remain debated: simple nephrostomy or double J drain. Ureteral-caliceal anastomosis is an interesting approach to rare surgical reoperations, particularly with major pyelo-ureteral dilatation. Laparoscopic surgery may prove usefulness in less small children. Endopyelotomy gives good results for post-operative stenoses.

[Desmoid tumors in an adolescent girl with familial adenomatous polyposis]. / Tumeurs desmoïdes dans le cadre d'une polypose adénomateuse familiale chez une adolescente.

Desmoid tumors (DT) are rare and nonmetastasizing fibroblastic neoplasms, characterized by local invasiveness. They occur sporadically or arise in the context of familial adenomatous polyposis (FAP; 5-10% of cases). Most cases develop sporadically in young adults, but some cases also occur in children. We report the case of an adolescent girl with FAP and DT, and we discuss the therapeutic strategies. An adolescent girl with FAP underwent surgery at the age of 14 years with total proctocolectomy. She had a neo-mutation in the APC gene at codon 1068, which is not usually associated with DT. Three years later, she had painful defecations. Imaging showed two abdominal DT. After a multidisciplinary team meeting, the patient was refused for surgery, and medical treatment with antihormonal agents and nonsteroidal anti-inflammatory drugs was started. Imaging 18 months later showed DT stabilization, but the patient had difficulties to control chronic pains, which required morphine treatment, hypnotic sessions, and transcutaneous electric nerve stimulation. This case highlights the importance of DT screening in patients with FAP, mainly after surgery, regardless of their age and genetic mutation. Progress remains to be made in determining DT risk factors and in developing treatment. DT are still difficult to cure because of their potential for local invasion and local recurrence, and need to be managed by a multidisciplinary team.

Left thoracoscopic thymectomy in children.

BACKGROUND: In the management of autoimmune myasthenia, thymectomy is recognized as effective surgical therapy. The necessity of complete radical thymectomy to achieve maximal improvement has been emphasized. Video-assisted thoracoscopic surgery has been successfully used for thymectomy in adults, and more recently in children, and has been described as achieving the same radicality and functional improvement as median sternotomy or as transcervical thymectomy. The aim of this work is to report our first thoracoscopic experience in this indication. METHODS: Patients with myasthenia gravis on anticholinesterase drugs and/or steroids are discussed for surgery in case of clinical deterioration despite increasing doses of medication or in case of no improvement. We decided to perform thoracoscopic thymectomies by a left-sided approach. Preoperative localization of thymic tissue is done by a thoracic CT exam. Patients are placed on their right side with a thoracic tilt under the thorax. Four thoracoscopic ports are used, a 10-mm for the camera and three 5-mm operating ports. The left lung was collapsed by selective intubation (double-lumen endotrachial intubation). RESULTS: Two boys, 7.5 and 14 years old, were addressed by the department of neurology for radical thymectomy. They presented an ocular myasthenia gravis for 2 years and a mild general myasthenia gravis for 7 years. The operative times were 120 and 240 min. There was no intraoperative or postoperative complication. Duration of thoracic drainage was 2 days. The children were discharged on the third postoperative day. For the second procedure, an ultrasound exam during surgery was necessary to localize the thymus exactly, thus enabling its complete resection without the need for a conversion. The follow-up is 19 and 7 months with a clinical improvement enabling the diminution of medication for both children, the end of ptosis for the first child, and the general improvement of muscle strength for the second. CONCLUSIONS: Thoracoscopic thymectomy in children with juvenile myasthenia gravis seems to offer a complete surgical resection, as do open techniques. In case of difficulties in finding the thymus, an ultrasound exam is feasible to enable complete resection. The left-sided thoracoscopic approach gives a good mediastinal and cervical exposition. Furthermore, being less painful in the postoperative period, it presents a less pronounced impairment of pulmonary function, and it presents good cosmetic effect.

[Prenatal ultrasound diagnosis of neuroblastoma]. / Neuroblastomes de diagnostic échographique anténatal.

With the progress of foetal ultrasonography, diagnosis of foetal tumors can be made before birth. We report five cases of prenatally detected neuroblastoma of the adrenal glands. Diagnosis was made during the third trimester in all cases. At birth no clinical sign related to the tumor was present, and urinary catecholamines were within normal ranges. On ultrasound scans the tumor was cystic in 1 case, solid in 2 cases and of mixed echogenicity in 2 cases. All children were operated on without pre or postoperative chemotherapy. In each setting the tumor was a stage I neuroblastoma according to the Evans classification. All children are alive and disease-free with a follow-up of 32 months at 14 years.

[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

Mediastinal pancreatic pseudocyst in a child. A thoracoscopic approach.

Mediastinal pancreatic pseudocyst is a rare occurrence in children and may be difficult to diagnose. Internal drainage via a cystenterostomy and a simple external drainage are two of the treatment options that are currently employed. We describe the case of an 11-year-old boy with a mediastinal pseudocyst who was treated via a thoracoscopic approach using an original pulmonary exclusion. The pseudocyst disappeared in 15 days and there has been no recurrence. No adjuvant treatment was necessary. Endoscopic retrograde cholangiopancreatography (ERCP) was useful in establishing the etiology (pancreas divisum). We believe that the thoracoscopic approach is a reproducible, simple, and safe procedure for the treatment of mediastinal pseudocysts. The technique may represent a valid alternative to cystogastrostomy.